The Committee met on October 17, 2006, from 12:30 p.m. until 5:30 p.m. in Minneapolis, Minnesota. The meeting was called to order by Dr. Jim Logan, chair, with vice chairman Dr. Joe D. Ross attending. There were 55 people in attendance. Committee members were welcomed and each introduced themselves.

Drs. Diane Sutton and Frank Ross, U.S. Department of Agriculture (USDA), Animal and Plant Health Inspection Service (APHIS), Veterinary Services (VS), Scrapie Program staff, presented the general Scrapie Program update. This report is in its entirety in these proceedings.

Dr. Chuck Gaiser, USDA-APHIS-VS, presented Descriptive Analysis and Scrapie Infected/Source Flocks and Investigations in FY 2006. This information is included in full in these proceedings.

Dr. Katherine O’Rourke, Agriculture Research Service (ARS), USDA, presented an ARS research update. The progress reported this year in the joint federal/state/industry scrapie eradication program is encouraging. ARS reported on progress in work conducted in Pullman, WA, on the issues of silent infection in genetically resistant ewes with natural exposure to scrapie, atypical scrapie, and goat scrapie. Genetically resistant (AAQR) ewes born to infected AAQQ dams have been moved to a secure permanent quarantine facility and bred to a susceptible (AAQQ) buck. All placentomes are examined for PrP-Sc using a number of biochemical tests and all genetically susceptible (AAQQ) lambs are held for observation for at least 18 months. No positive placentomes or lambs have been identified. The program moves into its final year and a final report will be given at next year’s meeting. A short update on atypical scrapie in Europe and the United Kingdom included information on genotypes found in the affected sheep and the types of diagnostic tests needed to identify the disease. If atypical scrapie is found in the United States, an additional control program may be necessary but it is likely that no changes in the current control program will be needed. Natural scrapie has been diagnosed in two goats using the rectal biopsy sampling procedure. These goats are being bred for examination of the placenta, transmission to kids, and distribution of the scrapie agent in the tissues and fluids. In addition, trials including breeding of goats following oral challenge with sheep scrapie and intracerebral challenge with goat scrapie have been initiated. In addition to placenta testing, assays for the TSE agent in urine and blood are in progress.

Dr. Katherine Marshall, Centers for Epidemiology and Animal Health (CEAH), VS-APHIS-USDA, presented information on Scrapie epidemiology, the rectal biopsy study, the goat scrapie slaughter prevalence study, and gave an update on the assessment of the scrapie program surveillance. Her presentation was titled Study to Evaluate Prion Protein Detection in Recto-anal Mucosa Associated Lymphoid Tissue (RAMALT) for Scrapie Diagnosis. Several recently published studies have shown that RAMALT tissue may be a useful test for field diagnosis of scrapie in live sheep and goats. This study will focus on the collection of rectal biopsy tissue in high-risk sheep and goats for up to 200 positive animals. Antemortem rectal biopsies and third eyelids will be collected, and within three weeks, animals will be necropsied for the collection of more rectal biopsy tissue, along with obex, retropharyngeal lymph nodes and tonsil tissue. We will then compare the sensitivity of the rectal biopsy tissue to third eyelid tissue in live animals, and to obex, retropharyngeal lymph nodes and tonsil tissues. We will also be looking at the repeatability of rectal biopsy results in ante- and post-mortem tissues from the same animal.

Scrapie Surveillance Update

Scrapie surveillance in the United States currently consists of the collection of tissue at slaughter and from animals that fall under non slaughter surveillance which includes samples collected from sheep and goats that are not in known positive or source flocks. These include clinical or dead animals from markets, renderers, diagnostic labs, farms, and feedlots, and third eyelid tests conducted on farms in black-faced sheep, and necropsies of high-risk animals. Approximately 105,116 samples have been collected at slaughter and 2,695 samples have been collected as part on non-slaughter surveillance. Since the beginning of the regulatory slaughter surveillance program in April 2003, there has been a reduction of scrapie in the black-faced sheep collected at slaughter. In 2006, a scrapie surveillance evaluation was conducted which provided recommendations to the scrapie eradication program for improving the efficiency of the program.

Caprine Scrapie Prevalence Study

This study will focus on the collection of two to five-year old goats at slaughter which we believe is the best opportunity for a wide array of the goat population, especially older ages. We will estimate prevalence of detectable scrapie in adult slaughter goats using a targeted sampling of goats which may have greater exposure to sheep and scrapie. Approximately 3,800 samples have been allocated to 79 slaughter plants in 20 states. Because of the fluctuation in adult goat slaughter, sample allocations may need to be adjusted based on feedback from the plants.

Dr. Jack Rhyan, VS-APHIS-USDA, presented preliminary results of the Evaluation of Five Prion Vaccines in the Mouse – Scrapie Model. Results of that study, conducted at the National Wildlife Research Center in Fort Collins by investigators John Pilon, Danelle Okeson, Lowell Miller, and Jack Rhyan and collaborators at the National Veterinary Services Laboratory (NVSL) in Ames, IA, showed three of the five peptide vaccines tried resulted in delayed onset of clinical signs of scrapie as compared to controls.. Two of the vaccines resulted in delayed onset of signs by two to three weeks; results were highly significant. Future studies will utilize the two most promising vaccines in mice, deer and sheep.

Dr. Cindy Wolf, University of Minnesota and Chair of the National Animal Identification System Sheep and Goat Identification working group presented information regarding sheep and goat identification and its correlation to the scrapie program identification requirements. The

scrapie program ID requirements have enabled the sheep and goat industries to progress with the National ID system with broad industry support. Although the scrapie system is not broadly using electronic identification, traceability is possible and the system is working. ID and recording movement in commerce and for exhibition is gaining support in the field. There is still concern and confusion amongst producers regarding the NAIS and there is a great need for continuing education about the purposes and need for animal identification.

Dr. Sutton and Marsh Koeneker, VS-APHIS-USDA, presented the scrapie program ID update and information on the emerging electronic technology being used for the Animal Health Surveillance Management and Mobile Information Management data in the scrapie program. A goal with this new technology is to "collect once and use many times" the necessary information and to minimize the potential for error in data entry.

The business portion of the meeting consisted primarily of discussion of six proposed changes to the Scrapie Uniform Methods and Rules (UMR). These proposed changes target surveillance, and identification compliance, and resulted in two resolutions being unanimously passed by the committee. One resolution requests USDA/APHIS and states to aggressively enforce the scrapie ID and record keeping requirements. The second resolution requests that development and implementation of an adequate surveillance system be a high priority of the USDA-APHIS-VS National Surveillance Unit.

The Committee considered four other proposed changes to the UMR and reached agreement to accept some minor changes and have Dr. Sutton incorporate them into the UM&R.

In Fiscal Year 2006 the Scrapie Eradication Program focused on: (1) cleaning up infected and source flocks utilizing a genetic based approach; (2) tracing and testing exposed animals and flocks; (3) expansion of regulatory slaughter surveillance (RSSS); (4) conducting consistent state reviews, (5) producer education and ID compliance; and (6) upgrading of the Scrapie National Database to provide web access through the Animal Health and Surveillance Monitoring (AHSM) website and to allow electronic transmission of test charts and results through a mobile information management module (MIMM).

Consistent State Reviews

States must meet the Consistent State requirements in 9 CFR 79.6 in order to move sheep and goats in interstate commerce with minimal restrictions. Forty-seven states have enacted the required identification rules. Regulatory action has been initiated to remove the remaining three states that are not in full compliance. Removal from the list would create a significant impact on the interstate movement of sheep and goats from those States. U.S. Department of Agriculture (USDA) is conducting onsite scrapie program consistent state reviews and has completed reviews in 35 states. The review of the remaining states will be completed by February 2007.

Scrapie Flock Certification Program

As of September 30, 2006, there were 2,027 flocks participating in the Scrapie Flock Certification Program (SFCP). Of these flocks 297 were certified flocks, 1,727 were complete monitored flocks, and 3 were selective monitored.

Infected and Source Flocks

As of September 30, 2006, there were 85 scrapie-infected and source flocks (48 infected and 37 source). There were a total of 116 new infected and source flocks reported for FY 2006. Figure 1 shows the number of new infected and source flocks by year. The total infected and source flock statuses that were released in FY 2006 was 100. A total of 343 positive scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL). Of these, 70 were RSSS cases, (collected in FY 2006 and confirmed in FY 2006 or FY 2007), and 222 positive field necropsy cases (most of these cases were found during depopulations of scrapie exposed animals in infected/source flocks), 14 necropsies of field cases retained long term for test evaluation, and 37 third eyelid regulatory tests confirmed in FY 2006. Three of the field cases were goats. One goat case, in Colorado, could not be linked to exposure in sheep as a result Colorado goats no longer meet the requirements to be classified as low-risk goats or low-risk commercial goats for interstate movement.

RSSS was designed based on the findings of the Center for Epidemiology and Animal Health (CEAH) Scrapie: Ovine Slaughter Surveillance (SOSS) study. The results of SOSS can be found at http://www.aphis.usda.gov/vs/ceah/cahm/Sheep/sheep.htm.

RSSS started April 1, 2003. It is a targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. During FY 2006, collections increased by 9% overall and by 16% for black and mottled faced sheep compared to FY 2005. Improvement in the overall program effectiveness and efficiency is demonstrated by the 33% decrease in percent positive black faced sheep compared to FY 2005 (0.67 to 0.45%, based on test results posted before November 6, 2006). During

FY 2006, 37,167 samples were collected. The distribution of these samples is shown in figure 2. There have been 70 NVSL confirmed positive cases that were collected in FY 2006. Face colors of these positives were 62 black and eight mottled. The percent positive by face color is shown in the figure 3 below.

Rare form of scrapie found in sheep; United States documents 1st case rare Nor98 scrapie strain----------------------------------------------------------------------------------The Wyoming Livestock Board says a sheep from a flock in the northeastern corner of the state has tested positive for a rare form of the disease scrapie, the 1st time the particular strain has been found in the United States.

The USDA Animal Plant Health Inspection Service [APHIS] notified the state Friday [16 Mar 2007] that the ewe tested positive for a form of scrapie consistent with the Nor98 strain, 1st diagnosed in Norway in 1998.

The livestock board said it doesn't expect the strain of the disease to become a major problem for the Wyoming sheep industry. State and federal officials intend to monitor the remainder of the flock, near the Black Hills, to make sure the disease doesn't become established.

According to a release from the livestock board, the ewe was slaughtered in Michigan as part of the USDA's regular scrapie slaughter surveillance program and traced back to the Wyoming flock.

The release states that the Nor98 strain of scrapie is rare even in Europe, with fewer than 300 cases diagnosed since it was identified in 1998.

Scrapie is a transmissible disease similar to chronic wasting disease found in deer and elk. Scrapie is limited to sheep and goats and takes years to affect an animal after it has been infected.

There are no known human health risks associated with scrapie.

"This provides evidence that the surveillance program is working," said Bryce Reece, executive director of the Wyoming Wool Growers Association. "It also indicates that the program is on the cutting edge of science to detect such a rare disease during standard surveillance." An epidemiological investigation is ongoing.

--Terry S. Singeltary Sr.

[Scrapie is a fatal, degenerative disease affecting the central nervous system of sheep and goats. It is among a number of diseases classified as transmissible spongiform encephalopathies (TSE). Infected flocks that contain a high percentage of susceptible animals can experience significant production losses. Over a period of several years, the number of infected animals increases, and the age at onset of clinical signs decreases, making these flocks economically unviable.

First recognized as a disease of sheep in Great Britain and other countries of Western Europe more than 250 years ago, scrapie has been reported throughout the world. Only 2 countries are recognized by the United States as being free of scrapie: Australia and New Zealand.

The 1st case of scrapie in the United States was diagnosed in 1947 in a Michigan flock. The flock owner had imported sheep of British origin through Canada for several years. APHIS conducted a slaughter surveillance study from 1 Apr 2002 to 31 Mar 2003, which determined the prevalence of scrapie in mature U.S. cull sheep to be 0.2 percent or one positive out of 500 cull sheep.

This particular strain was identified in Norway in 1998, in ARR/ARR genotype sheep. Some would argue this raises important issues with regard to control of scrapie infection in small ruminants. Of major concern, ARR/ARR sheep can no longer be regarded as free of natural TSE infection. The research on this particular strain can be viewed in its entirety at.Much of this paper was done on transgenic mice. This moderator contends that mice and sheep are a long way apart physiologically and everything found in a mouse is not necessarily the final word for research.

I respectfully request the final results of the mouse bio-assays test that were to have supposedly began 2+ years late, 5 years ago, on the imported sheep from Belgium ?

WHAT happened to the test results and MOUSE BIO-ASSAYS of those imported sheep from Belgium that were confiscated and slaughtered from the Faillace's, what sort of TSE did these animals have ?

WERE they atypical scrapie, BSE, and or typical scrapie ?

HOW much longer will you refuse to give us this information ? and for what reason ?

WHY is it that the Farm of the Mad Sheep of Mad River Valley were quarantined for 5 years, but none of these farms from Texas and Alabama with Atypical TSE in the Bovine, they have not been quarantined for 5 years, why not, with the real risk of BSE to sheep, whom is to say this was not BSE ?

FURTHERMORE, I respectfully request up front, that any fees for this FOIA be wavered due to the fact this information should be free to the public and is in the best interest for the public to have these final results, no financial gain from this FOIA information is to be made either. ...

I hope this finds you well. As you are aware I left the USDA lastyear. I can only update you on the sheep before that time. Contact wasestablished with the UK on doing the bioassay studies. They agreed.However, we were prioritized after their own needs, hence the delay. Iam aware that there are now additional labs in Europe running the mousebioassay strain typing. You will have to contact USDA for further word.

The Western blot tests on these animals were completed in April of thisyear. That means that we can begin the mouse inoculations. To get theresults of the Western blot tests, you will need to submit a Freedom ofInformation Act request through our FOIA office. The FAX number there is301-734-5941.

Thank you for you enquiry to the SEAC secretariat about mouse bioassayscommissioned by the USDA to investigate TSE cases in imported sheep.

After making a number of enquiries, it appears that Defra were not involvedwith this work. However, it is possible that a UK research laboratory wascontacted by the USDA about such tests but I have been unable to find outany further information. You may wish to make further enquiries with theUSDA.

have now received confirmation about my request from not only the OIG, but now have finally received response from APHIS saying thatthey have finally received my FOIA request for records relating to mouse bio-assays of sheep imported from Belgium, it's being processed, and that i should receive a response soon. ...well see what happens next///